TY - JOUR
T1 - Pediatric primary hyperparathyroidism
T2 - Surgical pathology and long-term outcomes in sporadic and familial cases
AU - Szabo Yamashita, Thomas
AU - Gudmundsdottir, Hallbera
AU - Foster, Trenton R.
AU - Lyden, Melanie L.
AU - Dy, Benzon M.
AU - Tebben, Peter J.
AU - McKenzie, Travis
N1 - Publisher Copyright:
© 2022 Elsevier Inc.
PY - 2023/4
Y1 - 2023/4
N2 - Background: Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce. Methods: Single-center retrospective review (1994–2020) of patients ≤21 years undergoing surgery for PHPT. Results: 66 patients were identified (61% female, 17 ± 3 years). 71% of patients were symptomatic at diagnosis. 32% of patients had known familial syndromes, most commonly MEN-1. 23% of patients without a known mutation had genetic testing, 22% positive. 56% of the total and 19% of the familial cohort underwent focused exploration. Single gland disease was found in 19% of familial vs 85% of sporadic cases, p < 0.00001. Persistence was 9%, all in the sporadic group, p = 0.11. Recurrence was 15%: 38% in the familial vs 2% in the sporadic groups, p=0.0004. Time to recurrence was 59 months (Q1-38, Q3-95), familial 61 vs 124 months sporadic, p=0.001. Conclusion: Pediatric PHPT is frequently sporadic, although 5% of apparent sporadic cases are secondary to syndromes. Familial cases have higher rates of recurrence, requiring closer follow-up.
AB - Background: Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce. Methods: Single-center retrospective review (1994–2020) of patients ≤21 years undergoing surgery for PHPT. Results: 66 patients were identified (61% female, 17 ± 3 years). 71% of patients were symptomatic at diagnosis. 32% of patients had known familial syndromes, most commonly MEN-1. 23% of patients without a known mutation had genetic testing, 22% positive. 56% of the total and 19% of the familial cohort underwent focused exploration. Single gland disease was found in 19% of familial vs 85% of sporadic cases, p < 0.00001. Persistence was 9%, all in the sporadic group, p = 0.11. Recurrence was 15%: 38% in the familial vs 2% in the sporadic groups, p=0.0004. Time to recurrence was 59 months (Q1-38, Q3-95), familial 61 vs 124 months sporadic, p=0.001. Conclusion: Pediatric PHPT is frequently sporadic, although 5% of apparent sporadic cases are secondary to syndromes. Familial cases have higher rates of recurrence, requiring closer follow-up.
KW - Familial hyperparathyroidism
KW - Hypercalcemia
KW - MEN
KW - Pediatric hyperparathyroidism
KW - Primary hyperparathyroidism
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U2 - 10.1016/j.amjsurg.2022.10.018
DO - 10.1016/j.amjsurg.2022.10.018
M3 - Article
C2 - 36270819
AN - SCOPUS:85140082906
SN - 0002-9610
VL - 225
SP - 699
EP - 702
JO - American journal of surgery
JF - American journal of surgery
IS - 4
ER -